Shy-Drager syndrome

Introduction to Shy-Drager Syndrome Shy-Drager syndrome (SDS) is a kind of multi-system atrophy disease with autonomic dysfunction. The most prominent symptom is that the patient's blood pressure is significantly reduced when the patient is in the upright position, causing insufficient blood supply to the whole brain. The patient is dizzy and dazzled. Even fainting. basic knowledge The proportion of illness: 0.001% Susceptible people: no special people Mode of infection: non-infectious Complications: ataxia

Causes of Shy-Drager syndrome

The disease is a degenerative disease of the central nervous system, the cause is unclear, the lesion involves the sympathetic nerve cells of the thoracolumbar medullary horn, the parasympathetic nerve cells of the brain stem and the medullary pulp, ganglion cells, pre- and post-ganglionic fibers, and the base. Sections, cerebellum and pyramidal tracts, electron microscopic observation of glial cells and neuronal nuclei in the lesions, cytoplasm, synapses in the presence of silver-containing inclusion bodies (inclusion bodies are linear, outsourced with a diameter of 20 ~ 30urn fluff The substance, consistent with the microtubule structure, is currently considered to be the basic pathological change of this syndrome.

Shy-Drager syndrome prevention

Usually pay attention to high salt and tyramine-rich diet, cultivate positive and cheerful personality, ensure adequate sleep, elderly and physically weak, should avoid prolonged bed rest, encourage and guide them to do rehabilitation exercises to maintain muscle tension and viscera The function is normal. It should be used with caution for dilating vasoactive drugs, sedatives, diuretics, etc. When the patient gets up or stands, the action should be slow. Those with severe hypotension should be treated with care to prevent fainting.

Complications of Shy-Drager syndrome Complications, ataxia

Accompanied by cerebellar ataxia, Parkinson's syndrome, dysfunction of cortical spinal cord and cortical medullary bundles, and muscle atrophy.

Symptoms of Shy-Drager Syndrome Common Symptoms Dizziness, dysphagia, nystagmus, gait, unstable blood pressure, decreased ataxia

According to typical clinical manifestations, middle-aged men, insidious onset, manifested symptoms of insufficient blood supply to the brain, such as dizziness or fainting, or accompanied by other autonomic symptoms, and extrapyramidal symptoms of the cone system.

(1) autonomic symptoms: orthostatic hypotension is a prominent manifestation, the patient's blood pressure is normal when lying down, but the blood pressure drops when standing, the systolic blood pressure can drop more than 30mmHg, the diastolic blood pressure can drop more than 20mmHg, the dizziness when standing is light, the head is heavy However, the fainting of the fainting, the most serious can only stay in bed for a long time.

Other autonomic symptoms include sexual dysfunction, impotence, sphincter dysfunction, constipation, diarrhea, urinary retention or incontinence, no sweat or sweat asymmetry, hoarseness, difficulty swallowing, and even sudden cardiac arrest.

(B) the symptoms of the nervous system: the cerebellar system is mainly affected by the cerebellar Shy-Drager syndrome (SDS-C) manifested as ataxia, gait instability, walking easy to fall, poorly constructed, neurological damage The basal ganglia is predominant, characterized by the symptoms and signs of typical Parkinson's disease, and the location of hypotension and autonomic symptoms, Parkinson's disease type Shy-Drager syndrome (SDS-P); Known as mixed type (SDS-M), the patient can be characterized by muscle rigidity, reduced movement, gait instability, tremor, nystagmus, hyperreflexia and positive pyramidal tract signs.

Examination of Shy-Drager syndrome

When standing upright, the blood pressure systolic pressure is lower than the lying position by 30mmHg, the heart rate is not accelerated, and the 24-hour urine norepinephrine and adrenaline discharge are low.

CT and MRI CT scans can be seen in some cases of pons, cerebellar progressive atrophy and no change in the supratentorial structure. MRI examination shows clearer brain stem and cerebellar atrophy. In SDS, high field strength MR/F2WI often shows bilateral The core signal is significantly reduced. This change can be preceded by the appearance of symptoms. It is generally thought to be caused by the pathological deposition of iron salts. However, the specific mechanism is unknown. It may be related to iron uptake by capillary endothelial cells during SDS. Related to transport disorders.

The sphincter electromyography (EMG) showed denervation and chronic neurogenic bladder. The EMg lesions of the bulbospongiosus and the external rectal sphincter were equal, and the bulbosus muscle reflex was normal in all patients.

Autonomic nerve function tests commonly used are sweat test, vasomotor test, various drug tests and Val-salva test, but its value in clinical diagnosis needs further study.

Diagnosis and differentiation of Shy-Drager syndrome

diagnosis

Diagnosis can be based on medical history, clinical symptoms, and laboratory findings.

Differential diagnosis

1. Simple syncope: There are obvious incentives, such as pain, fear, emotional stress, fatigue, etc., there are short-term prodromal symptoms before syncope, such as dizziness, nausea, paleness, sweating, etc.; syncope occurs most often in upright position or Sitting position, but no obvious orthostatic hypotension; blood pressure decreased when syncope, heart rate slowed and weak, pale and continued to the late syncope; recovery faster, no obvious aftereffects; no multiple autonomic and somatic nerve damage symptoms.

2. Urinary syncope: occurs at the end of urination or urination, causing a decrease in reflex blood pressure and syncope, most commonly when waking up in the middle of the night, which is different from the drop in blood pressure caused by changes in the position of the disease.

3. Cardiac syncope: Cardiovascular diseases, such as atrioventricular block, tachycardia, valvular heart disease, myocardial infarction, etc., can cause syncope due to reduced blood supply to the brain, causing syncope, abnormal ECG.

4. Carotid sinus syndrome: due to increased carotid sinus sensitivity, it can cause seizures when the carotid sinus is stimulated or compressed. When the carotid sinus pressure test is performed clinically, the heart rate can be slowed down and the blood pressure is lowered. Causes syncope.

5. Parkinson's disease: There are also extrapyramidal symptoms, and when they are the main manifestations, it is necessary to identify each other. Parkinson's disease has no orthostatic hypotension.

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